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systemic necrotizing vasculitis

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https://www.readbyqxmd.com/read/29209584/sudden-death-from-spontaneous-coronary-artery-dissection-due-to-polyarteritis-nodosa
#1
Cyrus M Munguti, Paul M Ndunda, Tabitha M Muutu
Spontaneous coronary artery dissection (SCAD) is an emerging and rare cause of acute coronary syndrome and sudden cardiac death. While it was previously reported among young females with fibromuscular dysplasia, new literature indicates that this condition could occur in older populations. Polyarteritis nodosa (PAN) causes systemic necrotizing vasculitis which typically affects small to medium-sized muscular arteries and could affect the coronary arteries. A few case reports of PAN causing acute coronary artery disease have been reported in the literature...
October 2, 2017: Curēus
https://www.readbyqxmd.com/read/29189887/eosinophilic-granulomatosis-with-polyangiitis-without-respiratory-symptoms-or-asthma-in-an-adolescent-case-report-and-literature-review
#2
REVIEW
Gülçin Otar Yener, Zahide Ekici Tekin, Neşe Çallı Demirkan, Selçuk Yüksel
Eosinophilic granulomatosis with polyangiitis (EGPA), also known as Churg-Strauss syndrome, is a systemic necrotizing vasculitis of the small and medium vessels. It is primarily associated with respiratory conditions such as asthma and sinusitis as well as eosinophilia, neuropathy, pulmonary infiltrates, and vasculitis. EGPA is extremely rare in the pediatric age group, and respiratory system disorders are usually predominant in EGPA patients. A 14-year-old boy presented with rash and severe extremity pain...
November 30, 2017: Rheumatology International
https://www.readbyqxmd.com/read/29139157/seronegative-granulomatosis-with-polyangiitis-presenting-with-multiple-cranial-nerve-palsies
#3
Eunhye Lee, Jynkyun Park, Seung Hong Choi, Sung-Hye Park
Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis (WG), is a rare systemic disease characterized by necrotizing granulomas and vasculitis that usually presents in the respiratory tract and renal system. Here, we report a case of anti-neutrophil cytoplasmic antibody (ANCA)-negative GPA with diagnostic difficulties. Neurologic involvement in GPA is not uncommon, with up to 50% of patients manifesting with neurologic defects; however, it is usually a late event. This patient was a 50-year-old man whose first manifestation was multiple neurologic defects without respiratory or renal symptoms...
November 14, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/29127573/eosinophilic-granulomatosis-with-polyangiitis-complicated-by-subarachnoid-hemorrhage-and-coronary-vasculitis-a-case-report-and-review-of-the-literature
#4
REVIEW
Shogo Matsuda, Shuzo Yoshida, Youhei Fujiki, Hidetoshi Satomi, Tohru Takeuchi, Yoshinobu Hirose, Shigeki Makino, Shigeki Arawaka
Eosinophilic granulomatosis with polyangiitis (EGPA) is characterized by necrotizing vasculitis of small-sized vessels with extravascular granulomas and eosinophilic infiltration. The case of a 48-year-old Japanese woman with EGPA, who presented concurrently with subarachnoid hemorrhage (SAH) and coronary vasculitis, is reported. She initially presented with bronchial asthma, and then 8 months later she developed various symptoms caused by systemic eosinophilic vasculitis and was admitted to our hospital. Three days after admission, she started oral corticosteroid therapy, and her 2009 Five-Factor Score (FFS) was 0...
November 10, 2017: Rheumatology International
https://www.readbyqxmd.com/read/29117463/vasculitis-and-iga-monoclonal-gammopathy-of-cutaneous-significance
#5
L Rousset, F Cordoliani, M Battistella, M Jachiet, E Pillebout, M Rybojad, M Bagot, E Oksenhendler, J-D Bouaziz
IgA vasculitis (Henoch-Schonlein purpura) is a small vessel systemic vasculitis involving IgA immunoglobulin polyclonal activation(1) . Monoclonal gammopathy of cutaneous significance is a subgroup of monoclonal gammopathy with skin disease, without myeloma or lymphoproliferative disorder(2) . We report 3 patients with vasculitis and IgA monoclonal gammopathy of cutaneous significance. Three men, 38, 37 and 50 years old, had a vascular purpura for more than 10 years with partial remission and frequent necrotic flares (Figure 1)...
November 8, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/29044308/heart-conduction-system-defects-and-sustained-ventricular-tachycardia-complications-in-a-patient-with-granulomatosis-with-polyangiitis-a-case-report-and-literature-review
#6
Laryssa Passos Sarmento Santos, Victor Guerreiro Bomfim, Camila Fagundes Bezerra, Natália Vieira Costa, Rafael Barreto Paes de Carvalho, Ricardo Sobral de Carvalho, Rogério da Hora Passos, Olivia Carla Bomfim Boaventura, André Luiz Nunes Gobatto
Granulomatosis with polyangiitis is a rare systemic inflammatory disorder characterized by vasculitis of the small arteries, the arterioles and the capillaries together with necrotizing granulomatous lesions. This case reports on a young female patient, previously diagnosed with granulomatosis with polyangiitis, who was admitted to the intensive care unit with seizures and hemodynamic instability due to a complete atrioventricular heart block. The event was associated with multiple episodes of sustained ventricular tachycardia without any structural heart changes or electrolyte disturbances...
July 2017: Revista Brasileira de Terapia Intensiva
https://www.readbyqxmd.com/read/29032100/-severity-of-illness-index-in-autoimmune-diseases-have-there-any-usefulness-in-medical-practice
#7
A Saunier, M-A Vandenhende, P Morlat, F Bonnet
INTRODUCTION: Assessing disease activity in patients suffering from autoimmune diseases is complex. Symptoms are multiple, often subjective and there are no reliable biomarkers. Many activity scores have been implemented to compare treatment efficacy in clinical trials. Their use in clinical practice is largely unknown. We performed a practical survey to analyze the use of activity scores in clinical practice to consider treatment response and to assess the determinants of their use. METHODS: A sample of French internists answered a questionnaire about activity scores of systemic lupus erythematosus, Sjögren's syndrome, autoimmune myositis and necrotizing vasculitis of small vessels...
November 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28992397/prognostic-significance-of-cavitary-lung-nodules-in-granulomatosis-with-polyangiitis-a-clinical-and-imaging-study-of-225-patients
#8
Bailey A Russell, Sindu Mohan, Rachandeep Chahal, Simon Carette, Christian Pagnoux
BACKGROUND: Granulomatosis with polyangiitis (GPA) is a systemic necrotizing vasculitis with pulmonary nodules as a common manifestation. Our study examined whether pulmonary nodules, and nodule type (solid versus cavitary), are associated with different disease manifestations and outcomes. METHODS: Demographic, clinical, biological, radiological data at diagnosis, during follow-up, and treatments of GPA patients followed at the Mount Sinai Hospital (Canada) vasculitis clinic were analyzed...
October 9, 2017: Arthritis Care & Research
https://www.readbyqxmd.com/read/28941467/an-atypical-presentation-of-cardiac-tamponade-and-periorbital-swelling-in-a-patient-with-eosinophilic-granulomatosis-with-polyangiitis-a-case-report
#9
Alexandra C Keefe, Joseph C Hymas, Lyska L Emerson, John J Ryan
BACKGROUND: Eosinophilic granulomatosis with polyangiitis is a rare, necrotizing systemic vasculitis associated with asthma and hypereosinophilia. Its cause and pathophysiology are still being elucidated. CASE PRESENTATION: We report a case of eosinophilic granulomatosis with polyangiitis in a 50-year-old Caucasian woman who presented with chest pain, dyspnea at rest, fever, and periorbital swelling. She was found to have significant hypereosinophilia and cardiac tamponade physiology...
September 24, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28690751/-ocular-beh%C3%A3-et
#10
Ahmed Alami, Mohamed Kriet, Karim Reda, Abdelkader Laktaoui, Abdelbaare Oubaaz
Our study aimed to determine the clinical, therapeutic and prognostic features of ocular involvement in patients with Behçet's disease treated in our Department of ophthalmology. We conducted a retrospective data collection from medical records of 20 patients treated at the military hospital in Laayoune. All patients underwent complete ophthalmological examination and fluorescein angiography if necessary. OCT exam was performed in two patients. Ten patients had anterior uveitis, complicated in one case by ocular hypertonia; two patients had intermediate uveitis; eight patients had posterior segment involvement complicated in one case by intravitreal hemorrhage...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28678392/adding-azathioprine-to-remission-induction-glucocorticoids-for-eosinophilic-granulomatosis-with-polyangiitis-churg-strauss-microscopic-polyangiitis-or-polyarteritis-nodosa-without-poor-prognosis-factors-a-randomized-controlled-trial
#11
RANDOMIZED CONTROLLED TRIAL
Xavier Puéchal, Christian Pagnoux, Gabriel Baron, Thomas Quémeneur, Antoine Néel, Christian Agard, François Lifermann, Eric Liozon, Marc Ruivard, Pascal Godmer, Nicolas Limal, Arsène Mékinian, Thomas Papo, Anne-Marie Ruppert, Anne Bourgarit, Boris Bienvenu, Loïck Geffray, Jean-Luc Saraux, Elisabeth Diot, Bruno Crestani, Xavier Delbrel, Laurent Sailler, Pascal Cohen, Véronique Le Guern, Benjamin Terrier, Matthieu Groh, Claire Le Jeunne, Luc Mouthon, Philippe Ravaud, Loïc Guillevin
OBJECTIVE: In most patients with nonsevere systemic necrotizing vasculitides (SNVs), remission is achieved with glucocorticoids alone, but one-third experience a relapse within 2 years. This study was undertaken to determine whether the addition of azathioprine (AZA) to glucocorticoids could achieve a higher sustained remission rate of newly diagnosed nonsevere eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA), microscopic polyangiitis (MPA), or polyarteritis nodosa (PAN)...
November 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28626961/comparing-polyarteritis-nodosa-in-children-and-adults-a-single-center-study
#12
Abdulsamet Erden, Ezgi D Batu, Hafize E Sönmez, Alper Sarı, Berkan Armagan, Zehra S Arıcı, Emre Bilgin, Umut Kalyoncu, Ömer Karadağ, Yelda Bilginer, Ali Ihsan Ertenli, Seza Özen
OBJECTIVE: Polyarteritis nodosa (PAN) is a necrotizing vasculitis of medium/small arteries. We aimed to examine the characteristics of adult- and childhood-onset PAN. METHODS: Fifteen pediatric (˂ 18 years) and 22 adult PAN patients who fulfilled the Ankara 2008 and American College of Rheumatology 1990 criteria, respectively, were included in the study. RESULTS: Five children had cutaneous and all the rest of the patients had systemic PAN...
August 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/28623488/vasculitis-for-the-internist-focus-on-anca-associated-vasculitis
#13
REVIEW
Benjamin Chaigne, Loïc Guillevin
Vasculitides comprise several diseases affecting vessels of different sizes, mainly arteries, but also capillaries and veins. Antineutrophil cytoplasm antibody-associated vasculitides (AAVs) belong to the systemic necrotizing small-vessel vasculitis group that comprises granulomatosis with polyangiitis, microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis. Internists often see AAV patients, and although their outcomes are usually good, the disease can be responsible for morbidity and mortality...
August 2017: Internal and Emergency Medicine
https://www.readbyqxmd.com/read/28592713/pulmonary-limited-granulomatosis-with-polyangiitis-coexisting-with-mixed-connective-tissue-disease
#14
Masaru Ando, Akihiko Goto, Mari Yamasue, Yuko Usagawa, Hiroaki Oka, Takehiko Shigenaga, Jun-Ichi Kadota
Granulomatosis with polyangiitis (GPA) is a systemic disease characterized by necrotizing, granulomatous vasculitis of the upper and lower respiratory tracts and glomerulonephritis, and is classified as a classical or limited form. The classical form of GPA demonstrates the involvement of the upper respiratory tract, sinuses, lungs and kidneys, whereas the limited form is characterized by the lack of the renal involvement with female predominance. On the other hand, mixed connective tissue disease (MCTD) shows the clinical and laboratorial features of systemic lupus erythematosus, systemic sclerosis and polymyositis, along with high titers of anti-ribonucleoprotein antibodies and is characterized by good response to corticosteroid therapy and favorable prognosis...
June 2017: Tohoku Journal of Experimental Medicine
https://www.readbyqxmd.com/read/28591026/imaging-diagnosis-of-orbital-wegener-granulomatosis-a-rare-case-report
#15
Bin Yang, Zhijian Yin, Shuai Chen, Feng Yuan, Wei Zhao, Yaying Yang
INTRODUCTION: Wegener granulomatosis (WG) is a rare idiopathic autoimmune disease causing necrotizing granulomatous vasculitis. Whether as the first symptom or as part of systemic changes, ocular manifestations in WG patients are not specific. Any part of the eyes can be affected, with the anterior segment and orbit most commonly involved. So, early diagnosis and treatment are essential for controlling the progression of the disease and improving the quality of life for patients. CLINICAL FINDINGS/PATIENT CONCERNS: Here we present a rare case of orbital WG of a 22-year-old woman was admitted to the hospital because of intense pain associated with decreased visual acuity in her right eye since 1 day...
June 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28587410/a-case-report-of-the-orbit-ocular-association-and-the-lung-in-granulomatosis-with-polyangiitis-a-diagnostic-challenge
#16
Cheng-Wei Lu, Xiu-Fen Liu, Ying Luan, Cheng-Bo Lu, Dan-Dan Zhou, Li-Min Guo, Ya-Bin Sun, Sheng-Nan Chen, Yun-Long Wu, Ji-Long Hao
Granulomatosis with polyangiitis (GPA) is a systemic form of vasculitis that involves small to medium sized vessels and is associated with high morbidity and mortality. GPA presents a continuous and difficult clinical diagnostic concern, due to the rarity of the disease and the diversity of the manifestations. This case report discusses the unusual symptoms presented by a particular patient, discusses these manifestations and explains how the final diagnosis was identified as GPA. A 40-year old Chinese woman was initially referred to the present institution for a progressive worsening pain, redness and gradual decrease in visual acuity in the eyes over the past 7-year period...
June 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28545345/systemic-necrotizing-polyarteritis-in-three-weaned-lambs-from-one-flock
#17
Mark Wessels, Ben Strugnell, Nick Woodger, Martin Peat, Severina Anna La Rocca, Akbar Dastjerdi
Systemic necrotizing polyarteritis was diagnosed in three 7-11-mo-old lambs from the same flock. Aneurysmal dilation and rupture of the gastroduodenal artery in 1 lamb resulted in fatal hemorrhage. All lambs had severe necrotizing vasculitis involving the small intestine, abomasum, mesentery, kidney, and heart with concurrent lymphocytic enteritis. Immunohistochemistry for T- and B-lymphocytes and macrophages found a T-cell- and macrophage-dominant transmural vascular infiltrate and T-cell-associated enteritis...
May 1, 2017: Journal of Veterinary Diagnostic Investigation
https://www.readbyqxmd.com/read/28540625/pituitary-dysfunction-in-granulomatosis-with-polyangiitis
#18
REVIEW
Daniela Esposito, Penelope Trimpou, Dario Giugliano, Mats Dehlin, Oskar Ragnarsson
PURPOSE: Granulomatosis with polyangiitis (GPA) is a multisystem disease, characterized by necrotizing small-vessel vasculitis, which mainly affects the respiratory tract and the kidneys. Pituitary involvement in GPA is rare, present in about 1% of all cases of GPA. To date, only case reports or small case series have been published. Herein we report clinical features, imaging findings, treatment and outcomes in three patients with GPA-related pituitary dysfunction (PD). METHODS: A retrospective analysis of three cases of GPA-related PD was conducted, followed by systematic review of the English medical literature using PubMed...
October 2017: Pituitary
https://www.readbyqxmd.com/read/28538395/palisaded-neutrophilic-and-granulomatous-dermatitis-as-a-novel-cause-of-hypercalcemia-a-case-report
#19
Michihito Kono, Tomoka Hasegawa, So Nagai, Toshio Odani, Kazumasa Akikawa, Yukiko Nomura, Hidetsugu Sato, Keisuke Kikuchi, Norio Amizuka, Hideaki Kikuchi
RATIONALE: Palisaded neutrophilic and granulomatous dermatitis (PNGD) is a benign, inflammatory dermatosis with distinct histopathological features often observed in patients with systemic diseases. There were no reports of PNGD without underlying systemic diseases as an underlying cause of hypercalcemia. Herein, we report a case of a 62-year-old man with hypercalcemia due to PNGD, but with no underlying systemic diseases, including tuberculosis, sarcoidosis, or vasculitis. PATIENT CONCERNS: Laboratory tests showed an elevated C-reactive protein level, an elevated corrected calcium level, a normal 25-hydroxyvitamin D level, and an elevated 1,25-dihydroxyvitamin D level...
May 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28508972/development-of-intracerebral-hemorrhage-in-the-short-term-clinical-course-of-a-patient-with-microscopic-polyangiitis-without-neurological-symptoms-at-diagnosis-an-autopsy-case
#20
Yoshia Miyawaki, Takayuki Katsuyama, Ken-Ei Sada, Kohei Taniguchi, Yuki Kakio, Jun Wada
A 77-year-old man with high-grade fever, progressive renal dysfunction, high serum level of C-reactive protein and positive serum myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA) was diagnosed with microscopic polyangiitis with rapidly progressive glomerulonephritis, and remission induction treatment with glucocorticoids and intravenous cyclophosphamide was initiated. Although his general condition improved in a short time, intracerebral hemorrhage occurred 12 days after the initiation of treatment and emergent hematoma evacuation was performed...
November 2016: CEN Case Reports
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